Association between habitual physical activity (HPA) and sleep quality in patients with cystic fibrosis

Purpose!#!Sleep disturbances and poor sleep quality are known to be present in patients with CF. Regular physical activity plays an important role in the treatment of CF patients due to its positive influence on progression of disease and quality of life. The aim of this work is to create a home-based sleep and activity profile and to investigate the influence of habitual physical activity (HPA) on sleep quality in children, adolescents, and adults with CF.!##!Methods!#!A total of 109 CF patients (64 male, mean age 22.7 ± 12.0 years; mean ppFEV1 63.0 ± 26.7) were equipped with an actigraph for a home-based collection of data on sleep and activity over 4 weeks.!##!Results!#!Age, FEV1, and BMI affect sleep and activity in CF patients. Especially younger age and higher FEV1 show a great influence on certain aspects of sleep (SE, TST, TIB, WASO, # of awakenings) and activity and its different intensities. General HPA does not affect sleep, but there is a strong correlation between times spent in vigorous to very vigorous intensities and better sleep quality.!##!Conclusion!#!Besides younger age and higher FEV1, daily activity in higher intensities influences sleeping behavior of CF patients in a positive way. Patients with poor sleep quality and sleep disturbances possibly benefit from an intensification of physical activity in the home environment. TRAIL REGISTRATION: number: 14-6117-BO (University Duisburg-Essen) and NCT03518697 (clinical trials)

Die induzierbare laryngeale Obstruktion (ILO)

Wiederholt episodenhaft auftretende Atemnot ist im Kindes- und Jugendalter ein häufiges Symptom. Neben anstrengungsinduzierter Bronchialobstruktion im Rahmen eines Asthma bronchiale ist eine funktionelle Genese eine sehr häufige Differenzialdiagnose. Dennoch wird diese Diagnose häufig nicht oder mit langer Latenz gestellt. Unter dem Oberbegriff "ILO" ("inducible laryngeal obstruction") werden sowohl funktionelle supraglottische Obstruktionen durch Kollaps der Knorpelstrukturen als auch Dysfunktionen auf Glottisebene wie "vocal cord dysfunction" (VCD) subsumiert. Körperliche Anstrengung ist ein häufiger Auslöser; es werden jedoch auch Beschwerdebilder ohne Anstrengungsbezug beobachtet. Es wird der Erkenntnisstand zur Pathophysiologie referiert und die klinische Präsentation beschrieben. Ein wesentlicher Fokus des Artikels liegt im Folgenden auf der Darstellung eines sinnvollen und Ressourcen-orientierten diagnostischen Vorgehens. Der CLE-Test ("continuous laryngoscopy exercise test") als Provokationsmethode unter Wach-Videolaryngoskopie ist der Goldstandard, jedoch wird diese Diagnostik im deutschsprachigen Raum nicht flächendeckend vorgehalten. Dieses Positionspapier stellt daher die diagnostische Wertigkeit verschiedener anderer Diagnostik-Algorithmen und anamnestischer Informationen heraus. Ein weiterer Schwerpunkt des Papiers besteht in der detaillierten Vorstellung geeigneter atemphysiotherapeutischer Interventionen.Episodically occurring dyspnea has a high prevalence in childhood and adolescence. Besides exercise-induced bronchial obstruction in a child with asthma, functional respiratory problems are also frequently encountered; however, in cases of functional disorders the correct diagnosis is often not made or made after a long latent period. The term inducible laryngeal obstruction (ILO) comprises functional supraglottic obstruction due to cartilage collapse as well as glottic disorders, such as vocal cord dysfunction (VCD). Physical exertion is a common trigger but there are also other entities which occur without relation to exercise. One main goal of the article is to delineate a reasonable diagnostic pathway taking the limited access to a continuous laryngoscopy exercise (CLE) test in Germany into account: The CLE test is presently done by a treadmill provocation test while visualizing the larynx with a direct laryngoscopy; however, this procedure is not widely available in Germany. Hence the authors of this paper discuss the diagnostic power of other procedures and suggest a reasonable diagnostic algorithm. A second focus of the paper is on a detailed presentation and discussion of suitable physiotherapeutic interventions

Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF RegistryResearch in context

Summary: Background: Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) improves multiple clinical outcomes in people with cystic fibrosis (pwCF) with at least one F508del allele. This study evaluated the real-world impact of ETI on lung function, nutritional status, pulmonary exacerbation frequency, and sweat chloride concentrations in a large group of pwCF. Methods: This observational cohort study used data from the German CF Registry for pwCF who received ETI therapy and were followed up for a period of 12 months. Findings: The study included 2645 pwCF from 67 centres in Germany (mean age 28.0 ± 11.5 years). Over the first year after ETI was initiated, percent predicted forced expiratory volume in 1 s (ppFEV1) increased by 11.3% (95% confidence interval [CI] 10.8–11.8, p < 0.0001), body mass index (BMI) z-score increased by 0.3 (95% CI 0.3–0.4, p < 0.0001) in individuals aged 12 to <18 years and BMI in adults increased by 1.4 kg/m2 (95% CI 1.3–1.4, p < 0.0001), pulmonary exacerbations decreased by 75.9% (p < 0.0001) and mean sweat chloride concentration decreased by 50.9 mmol/L (95% CI –52.6, −49.3, p < 0.0001). Improvements in ppFEV1 over the first year of therapy were greater in pwCF who had not previously received cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy (12.6% [95% CI 11.9–13.4] vs. 9.7% [95% CI 9.0–10.5] in those with prior CFTR modulator treatment. Interpretation: These real-world data are consistent with the findings of randomised clinical trials, and support the use of ETI as a highly effective treatment option for pwCF who have at least one F508del allele. Funding: None